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Tg(tetO-DBT)A1Geh
Transgene Detail
Nomenclature
Symbol: Tg(tetO-DBT)A1Geh
Name: transgene insertion A1, Gregg E Homanics
MGI ID: MGI:3704903
Synonyms: TRE-E2 (line A)
Transgene: Tg(tetO-DBT)A1Geh  Location: unknown  
Transgene
origin
Strain of Origin:  C57BL/6J
Transgene
description
Transgene Type:    Transgenic (Humanized sequence, Hypomorph, Inducible, Inserted expressed sequence)
Mutation:    Insertion
 
Tg(tetO-DBT)A1Geh expresses 1 gene
 
Mutation detailsThe TRE-E2 (or tetO-DBT) transgene contains a tetracycline response element and minimal human CMV promoter controlling a human E2 subunit (DBT gene) cDNA which has been modified to contain a 4x alanine linker followed by a c-myc epitope tag at the carboxy terminus to facilitate detection. Levels of the human E2 protein are approximately equal to mouse E2 protein in livers of control mice. However, when crossed to animals that express a transgene encoding the tetracycline regulated transactivator protein (Tg(tTALap)5Bjd) and are homozygous for a knockout allele of mouse E2 (Dbttm1Geh) are treated with doxycycline, the E2 protein levels correlate with only ~5-6% of normal branched-chain keto acid dehydrogenase (BCKDH) activity. It is one hypothesis that addition of the c-myc tag interferes with BCKDH subunit assembly (ie. interaction the human E2 subunit with mouse E1 and E3 complexes), thus reducing enzymatic activity. (J:119973)
Phenotypes
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View phenotypes for all genotypes (concatenated display).
Disease models
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Find Mice (IMSR)
Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
Carrying this Mutation:  Mouse Strains: 1 strain available      Cell Lines: 0 lines available
References
Original:  J:119973 Homanics GE, et al., Production and characterization of murine models of classic and intermediate maple syrup urine disease. BMC Med Genet. 2006;7:33
All:  2 reference(s)

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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
07/28/2015
MGI 5.22
The Jackson Laboratory